Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases.
OBJECTIVE
The purpose of this study was to describe the clinical characteristics of a novel group of patients with obsessive-compulsive disorder (OCD) and tic disorders, designated as pediatric autoimmune neuropsychiatric disorders associated with streptococcal (group A beta-hemolytic streptococcal [GABHS]) infections (PANDAS).


METHODS
The authors conducted a systematic clinical evaluation of 50 children who met all of the following five working diagnostic criteria: presence of OCD and/or a tic disorder, prepubertal symptom onset, episodic course of symptom severity, association with GABHS infections, and association with neurological abnormalities.


RESULTS
The children's symptom onset was acute and dramatic, typically triggered by GABHS infections at a very early age (mean = 6.3 years, SD = 2.7, for tics; mean = 7.4 years, SD = 2.7, for OCD). The PANDAS clinical course was characterized by a relapsing-remitting symptom pattern with significant psychiatric comorbidity accompanying the exacerbations; emotional lability, separation anxiety, nighttime fears and bedtime rituals, cognitive deficits, oppositional behaviors, and motoric hyperactivity were particularly common. Symptom onset was triggered by GABHS infection for 22 (44%) of the children and by pharyngitis (no throat culture obtained) for 14 others (28%). Among the 50 children; there were 144 separate episodes of symptom exacerbation; 45 (31%) were associated with documented GABHS infection, 60 (42%) with symptoms of pharyngitis or upper respiratory infection (no throat culture obtained), and six (4%) with GABHS exposure.


CONCLUSIONS
The working diagnostic criteria appear to accurately characterize a homogeneous patient group in which symptom exacerbations are triggered by GABHS infections. The identification of such a subgroup will allow for testing of models of pathogenesis, as well as the development of novel treatment and prevention strategies.
Swedo
S E
SE
Section on Behavioral Pediatrics, NIMH, Rockville Pike, Bethesda, MD 20892-1381, USA. swedos@irp.nimh.nih.gov
Leonard
H L
HL
Garvey
M
M
Mittleman
B
B
Allen
A J
AJ
Perlmutter
S
S
Lougee
L
L
Dow
S
S
Zamkoff
J
J
Dubbert
B K
BK
D000208:Acute Disease; D017668:Age of Onset; D001289:Attention Deficit Disorder with Hyperactivity / Q000175:diagnosis / Q000453:epidemiology; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000209:etiology / Q000276:immunology; D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology; D002648:Child; D002653:Child Behavior Disorders / Q000175:diagnosis / Q000453:epidemiology; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D006801:Humans; D008297:Male; D001523:Mental Disorders / Q000175:diagnosis / Q000453:epidemiology; D009771:Obsessive-Compulsive Disorder / Q000175:diagnosis* / Q000453:epidemiology / Q000276:immunology; D010612:Pharyngitis / Q000175:diagnosis / Q000209:etiology / Q000276:immunology; D012141:Respiratory Tract Infections / Q000175:diagnosis / Q000209:etiology / Q000276:immunology; D012720:Severity of Illness Index; D013290:Streptococcal Infections / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D013297:Streptococcus pyogenes / Q000276:immunology*; D013981:Tic Disorders / Q000175:diagnosis* / Q000209:etiology